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2014 ; 26
(3
): 320-7
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Interstitial lung disease in children
#MMPMID24752172
Kuo CS
; Young LR
Curr Opin Pediatr
2014[Jun]; 26
(3
): 320-7
PMID24752172
show ga
PURPOSE OF REVIEW: There has been tremendous progress in the approach to
childhood interstitial lung diseases (chILD), with particular recognition that
interstitial lung disease (ILD) in infants is often distinct from the forms that
occur in older children and adults. Diagnosis is challenging because of the
rarity of ILD and the fact that the presenting symptoms of ILD often overlap
those of common respiratory disorders. This review summarizes the newly published
recommendations for diagnosis and management, and highlights the recent
scientific advances in several specific forms of chILD. RECENT FINDINGS: Clinical
practice guidelines emphasize the role for chest computed tomography, genetic
testing, and lung biopsy in the diagnostic evaluation of children with suspected
ILD. Recent studies have better defined the characteristics and molecular
understanding of several different forms of ILD, including neuroendocrine cell
hyperplasia of infancy and ILD, due to mutations in genes affecting surfactant
production and metabolism. Despite significant progress, definitive therapies are
often lacking. SUMMARY: chILD encompasses a collection of rare, diffuse lung
diseases. Timely recognition of children with suspected ILD and initiation of
appropriate diagnostic evaluations will facilitate medical management. Systematic
approaches to clinical care and further studies are needed to improve the
outcomes of children with these rare disorders.